Definition

Sickle cell anemia is a genetic disorder. It alters the shape of the red blood cells (RBCs). This decreases their ability to carry oxygen. It can also cause acute episodes of pain. These are called a sickle cell crisis. The body will also destroy the sickle cells. The loss of RBCs results in
anemia
.

The change in the ability and number of RBCs can decrease the amount of oxygen for the body.

Red Blood Cells: Normal and Sickle

Causes

Sickle cell disease is a genetic disorder. If you receive one defective gene from each of your parents, then you will have sickle cell disease. If you only have one defective gene, you are said to have sickle cell trait, but not sickle cell disease. Although you won’t usually have any symptoms, you can pass this gene on to your children.

Risk Factors

Factors that increase your chance for sickle cell anemia include:

Race: Blacks of sub-Saharan origin

Ethnicity: Greeks, Italians, and people from some parts of India, Central and South America, and the Arabian Peninsula

Symptoms

This condition produces a group of symptoms known as a sickle cell crisis. These are episodes of pain that occur with varying frequency and severity. It is usually followed by periods of remission. The risk for a sickle cell crisis increases with any activity that boosts the body's requirement for oxygen. This may include illness, physical or emotional
stress, or high altitudes.

These painful crises can last hours or days. They affect the bones of the back, the long bones, and the chest. The crises can be severe enough to require hospital admission for pain control and IV fluids.

Treatment

Penicillin

Newborns with sickle cell disease may be given penicillin . It is given twice a day, beginning at age two months. It is continued until at least five years of age.

Pneumococcal Vaccine

It is recommended that children with sickle cell disease who are 2-5 years of age receive
pneumococcal vaccine (PCV-7)
. Other routine immunizations should also be given, such as an annual flu shot.

Hydroxyurea
(Hydrea)

Hydroxyurea is the first drug to significantly prevent complications of sickle cell disease. It increases the production of fetal hemoglobin. This decreases the number of deformed RBCs. As a result, it reduces the frequency of sickle cell crisis. Hydrea is not appropriate for everyone with sickle cell disease.

Blood Transfusions

Blood transfusions can treat and prevent some of the complications. Transfusion therapy can help prevent recurring strokes in children.

Bone Marrow Transplant

A
bone marrow transplant
from a matched donor may be effective. There are medical risks involved. Recipients must also take drugs that suppress the immune system for the rest of their lives.

If you or your child is diagnosed with sickle cell anemia, follow your doctor's
instructions
.

Prevention

Sickle cell anemia cannot be prevented. There are some general guidelines that may keep the condition under control. Be sure to discuss these with your doctor:

CANADIAN RESOURCES:

References:

Furie KL, Kasner SE, et al. Guidelines for the Prevention of Stroke in Patients With Stroke or Transient Ischemic Attack: A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association.
Stroke
. 2010 October 21.

Sickle Cell Disease. American Academy of Family Physicians' Family Doctor.org website. Available at:
http://familydoctor.org/familydoctor/en/diseases-conditions/sickle-cell-disease.html. Updated January 2011. Accessed July 1, 2013.

What is sickle cell anemia?
National Heart, Lung, and Blood Institute
website. Available at:
http://www.nhlbi.nih.gov/health/health-topics/topics/sca/.
Updated September 28, 2012. Accessed July 1, 2013.

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